What are the effects of red blood cell transfusions for preventing silent cerebral infarcts among children with sickle cell disease?

2017 ◽  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Cell Disease ◽  
Cerebral Infarcts ◽  
Red Blood Cell Transfusions

scholarly journals Red blood cell alloimmunization in sickle cell disease: pathophysiology, risk factors, and transfusion management

Blood ◽  
2012 ◽  
Vol 120 (3) ◽  
pp. 528-537 ◽  
Author(s):  
Karina Yazdanbakhsh ◽  
Russell E. Ware ◽  
France Noizat-Pirenne
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Cell Disease ◽  
Factors Associated ◽  
Life Threatening ◽  
Red Blood Cell Transfusions ◽  
Transfusion Reactions

Abstract Red blood cell transfusions have reduced morbidity and mortality for patients with sickle cell disease. Transfusions can lead to erythrocyte alloimmunization, however, with serious complications for the patient including life-threatening delayed hemolytic transfusion reactions and difficulty in finding compatible units, which can cause transfusion delays. In this review, we discuss the risk factors associated with alloimmunization with emphasis on possible mechanisms that can trigger delayed hemolytic transfusion reactions in sickle cell disease, and we describe the challenges in transfusion management of these patients, including opportunities and emerging approaches for minimizing this life-threatening complication.

Leukoreduced red blood cell transfusions do not induce platelet glycoprotein antibodies in patients with sickle cell disease

Transfusion ◽  
2016 ◽  
Vol 56 (9) ◽  
pp. 2267-2273 ◽  
Author(s):  
Robert Sheppard Nickel ◽  
Anne M. Winkler ◽  
John T. Horan ◽  
Jeanne E. Hendrickson
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Platelet Glycoprotein ◽  
Cell Disease ◽  
Red Blood Cell Transfusions

scholarly journals Red blood cell transfusions are associated with HLA class I but not H-Y alloantibodies in children with sickle cell disease

2015 ◽  
Vol 170 (2) ◽  
pp. 247-256 ◽  
Author(s):  
Robert S. Nickel ◽  
Jeanne E. Hendrickson ◽  
Marianne M. Yee ◽  
Robert A. Bray ◽  
Howard M. Gebel ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Hla Class I ◽  
Class I ◽  
Cell Disease ◽  
Red Blood Cell Transfusions

Matched Related Donor Transplants for Sickle Cell Disease: Report from the Center for International Blood and Transplant Research.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 2030-2030
Author(s):  
Julie A. Panepinto ◽  
Jeanette Carreras ◽  
Mark Walters ◽  
Mary Eapen ◽  
Judith Marsh ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Recurrent Disease ◽  
Chronic Gvhd ◽  
Disease Free Survival ◽  
Disease Recurrence ◽  
Cell Disease ◽  
Red Blood Cell Transfusions

Abstract Allogeneic hematopoietic cell transplantation (HCT) is the only known therapy to cure sickle cell disease though few patients receive this therapy. We report outcomes after HLA-matched sibling HCT in 68 patients with sickle cell disease, transplanted in 1989 to 2002. Of these, 33 (49%) were transplanted between 1999 and 2002. Median age at transplantation was 10 (range 2–30) years. Hemoglobin SS was the predominant genotype. Indications for HCT were predominately stroke (n=24) and recurrent vaso-occlusive crises (n=24); others included acute chest syndrome, increasing transfusion requirement, progressive iron overload and recurrent priapism. Forty-four patients (66%) received ≥ 10 red blood cell transfusions prior to HCT. Twenty patients (26%) had poor performance scores prior to transplantation. Busulfan and cyclophosphamide was the most frequently used conditioning regimen (n=63, 92%). Fifty-five patients (81%) received bone marrow allografts, 9 patients (13%) received mobilized peripheral blood, 3 patients (4%) received umbilical cord blood, and 1 patient received umbilical cord blood and bone marrow from the same donor. All patients achieved neutrophil recovery and the probability of platelet recovery ≥20,000/ul at day 100 was 93% (95% confidence interval [CI], 86–98)%. The probabilities of grades 2–4 acute graft-versus-host disease (GVHD) at day 100 and chronic GVHD at 5 years were 10% (95% CI, 4–19%) and 22% (95% CI, 12–33%), respectively. Sixty-five of 68 patients are alive after HCT with a median follow up of 5 years. The 5-year probabilities of overall and disease-free survival were 97% (95% CI, 88–100%) and 84% (95% CI, 75–95%), respectively. We defined treatment failure (inverse of disease-free survival) as death from any cause or disease recurrence defined as having a hemoglobin S >50%. Recurrent disease was the predominant cause of treatment failure (n=10). Of these 10 patients, 8 had return of clinical symptoms and the remaining 2 were symptom-free. Among patients with recurrent disease, 5 had received >10 red blood cell transfusions pre-transplant and 1 was reported to have red blood cell alloantibodies. Four patients with recurrent disease were transplanted for stroke and 6 for vaso-occlusive crisis. Nine of 10 patients received busulfan and cyclophosphamide for their conditioning and the remaining patient, total body irradiation 200cGy (single fraction), fludarabine and anti-thymocyte globulin. Three patients died; all deaths occurred more than 100 days after HCT. Causes of death were hemorrhage (n=1), multi-organ failure (n=1) and unknown (n=1). Of the 10 patients with stroke that had magnetic resonance imaging (MRI) of the brain pre- and post-transplant, 8 showed stable disease post transplant, one showed improvement and one had a worsening MRI. Overall survival after HCT for sickle cell disease is excellent, however recurrent disease and chronic GVHD remain a concern. Future studies should focus on strategies aimed at reducing disease recurrence.

scholarly journals Hyperhemolysis Syndrome in Patients With Sickle Cell Disease

2018 ◽  
Vol 142 (11) ◽  
pp. 1425-1427 ◽  
Author(s):  
Mara Banks ◽  
James Shikle
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Genetic Disease ◽  
Blood Products ◽  
Cell Disease ◽  
Laboratory Findings ◽  
Red Blood Cell Transfusions ◽  
Compatible Blood

Sickle cell disease is a genetic disease commonly affecting people of African, Indian, and Mediterranean descent. Patients with this chronic disease often require lifelong red blood cell transfusions. Formation of alloantibodies and autoantibodies are well-known complications that can arise with multiple transfusions. Another rare, but serious complication associated with transfusion is hyperhemolysis syndrome. The acquisition of new and/or rare alloantibodies can make it more difficult to find compatible blood products for patients with sickle cell disease. Genotyping and national donor registries are useful tools for finding appropriate blood products for these patients. This review will describe the clinical and laboratory findings of sickle cell disease, including hyperhemolysis syndrome. The challenges associated with locating compatible blood for patients with various red blood cell antibodies will be reviewed.

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